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Bilateral apical pulmonary fibrosis

MedGen UID:
1778266
•Concept ID:
C5539667
•
Disease or Syndrome
HPO: HP:0033539

Definition

A reticular pattern of linear or lineonodular densities in apical portions of both the right and left lungs seen initially on high-resolution computed tomography and in case of progression also on standard chest x-ray. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBilateral apical pulmonary fibrosis

Recent clinical studies

Etiology

Application of Routine Echocardiography Combined with Contrast-Enhanced Echocardiography in the Diagnosis of Endomyocardial Fibrosis.
Wu B, Yang Y, Zhao P
Discov Med 2023 Dec;35(179):1052-1063. doi: 10.24976/Discov.Med.202335179.101. PMID: 38058070
Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease.
Enomoto Y, Nakamura Y, Colby TV, Johkoh T, Sumikawa H, Nishimoto K, Yoshimura K, Matsushima S, Oyama Y, Hozumi H, Kono M, Fujisawa T, Enomoto N, Inui N, Iwashita T, Suda T
PLoS One 2017;12(6):e0180283. Epub 2017 Jun 30 doi: 10.1371/journal.pone.0180283. PMID: 28666014Free PMC Article
Development of chronic pulmonary aspergillosis in adult asthmatics with ABPA.
Lowes D, Chishimba L, Greaves M, Denning DW
Respir Med 2015 Dec;109(12):1509-15. Epub 2015 Sep 12 doi: 10.1016/j.rmed.2015.09.007. PMID: 26507434
Bronchiectasis-related amyloidosis as a cause of chronic renal failure.
Akçay S, Akman B, Ozdemir H, Eyüboğlu FO, Karacan O, Ozdemir N
Ren Fail 2002 Nov;24(6):815-23. doi: 10.1081/jdi-120015683. PMID: 12472203
Spontaneous pneumothorax in patients irradiated for Hodgkin's disease and other malignant lymphomas.
Pezner RD, Horak DA, Sayegh HO, Lipsett JA
Int J Radiat Oncol Biol Phys 1990 Jan;18(1):193-8. doi: 10.1016/0360-3016(90)90284-q. PMID: 2298622

Diagnosis

Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis.
Tetikkurt C, Kubat B, Kulahci C, Tetikkurt S, Caliskaner Ozturk B
Monaldi Arch Chest Dis 2021 Apr 6;91(3) doi: 10.4081/monaldi.2021.1713. PMID: 33840184
Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature.
Ali MS, Ramalingam VS, Haasler G, Presberg K
BMJ Case Rep 2019 Apr 20;12(4) doi: 10.1136/bcr-2019-229402. PMID: 31005872Free PMC Article
Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease.
Enomoto Y, Nakamura Y, Colby TV, Johkoh T, Sumikawa H, Nishimoto K, Yoshimura K, Matsushima S, Oyama Y, Hozumi H, Kono M, Fujisawa T, Enomoto N, Inui N, Iwashita T, Suda T
PLoS One 2017;12(6):e0180283. Epub 2017 Jun 30 doi: 10.1371/journal.pone.0180283. PMID: 28666014Free PMC Article
Chronic Cough and Bilateral Pneumothoraces in a Nonsmoker.
O'Beirne SL, Escalon JG, Arkin JE, Stiles BM, Kaner RJ, Legasto AC, Narula N, King TC
Chest 2016 Feb;149(2):e49-e55. doi: 10.1016/j.chest.2015.10.070. PMID: 26867855
Pulmonary manifestations of ankylosing spondylitis.
Kanathur N, Lee-Chiong T
Clin Chest Med 2010 Sep;31(3):547-54. doi: 10.1016/j.ccm.2010.05.002. PMID: 20692546

Therapy

Unilateral Upper Lung Field Pulmonary Fibrosis Radiologically Consistent with Pleuroparenchymal Fibroelastosis after Thoracotomy: A New Disease Entity Related to Thoracotomy.
Sekine A, Satoh H, Iwasawa T, Matsui K, Ikeya E, Ikeda S, Yamakawa H, Okuda R, Kitamura H, Shinohara T, Baba T, Komatsu S, Kato T, Hagiwara E, Ogura T
Respiration 2017;94(5):431-441. Epub 2017 Sep 1 doi: 10.1159/000479331. PMID: 28858871
A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone.
Sato S, Hanibuchi M, Takahashi M, Fukuda Y, Morizumi S, Toyoda Y, Goto H, Nishioka Y
Intern Med 2016;55(5):497-501. Epub 2016 Mar 1 doi: 10.2169/internalmedicine.55.5047. PMID: 26935370
Long-term follow-up of pulmonary function in chronic eosinophilic pneumonia. Groupe d'Etude en Pathologie Interstitielle de la Société de Pathologie Thoracique du Nord.
Durieu J, Wallaert B, Tonnel AB
Eur Respir J 1997 Feb;10(2):286-91. doi: 10.1183/09031936.97.10020286. PMID: 9042622
Tuberculosis in liver transplant patients.
Meyers BR, Halpern M, Sheiner P, Mendelson MH, Neibart E, Miller C
Transplantation 1994 Aug 15;58(3):301-6. PMID: 8053051
Spontaneous pneumothorax in patients irradiated for Hodgkin's disease and other malignant lymphomas.
Pezner RD, Horak DA, Sayegh HO, Lipsett JA
Int J Radiat Oncol Biol Phys 1990 Jan;18(1):193-8. doi: 10.1016/0360-3016(90)90284-q. PMID: 2298622

Prognosis

Hermansky-Pudlak syndrome-associated pneumothorax with rapid progression of respiratory failure: a case report.
Kato Y, Kato M, Ihara H, Hayakawa E, Shibayama K, Miura K, Yamada T, Mitsuishi Y, Shukuya T, Ito J, Matsunaga T, Sato T, Suzuki K, Takahashi K
BMC Pulm Med 2020 Oct 6;20(1):259. doi: 10.1186/s12890-020-01302-8. PMID: 33023548Free PMC Article
Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study.
Enomoto Y, Nakamura Y, Satake Y, Sumikawa H, Johkoh T, Colby TV, Yasui H, Hozumi H, Karayama M, Suzuki Y, Furuhashi K, Fujisawa T, Enomoto N, Inui N, Iwashita T, Kuroishi S, Yokomura K, Koshimizu N, Toyoshima M, Imokawa S, Yamada T, Shirai T, Hayakawa H, Suda T
Respir Med 2017 Dec;133:1-5. Epub 2017 Nov 6 doi: 10.1016/j.rmed.2017.11.003. PMID: 29173442
Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease.
Enomoto Y, Nakamura Y, Colby TV, Johkoh T, Sumikawa H, Nishimoto K, Yoshimura K, Matsushima S, Oyama Y, Hozumi H, Kono M, Fujisawa T, Enomoto N, Inui N, Iwashita T, Suda T
PLoS One 2017;12(6):e0180283. Epub 2017 Jun 30 doi: 10.1371/journal.pone.0180283. PMID: 28666014Free PMC Article
Pulmonary manifestations of ankylosing spondylitis.
Kanathur N, Lee-Chiong T
Clin Chest Med 2010 Sep;31(3):547-54. doi: 10.1016/j.ccm.2010.05.002. PMID: 20692546
Spontaneous pneumothorax in patients irradiated for Hodgkin's disease and other malignant lymphomas.
Pezner RD, Horak DA, Sayegh HO, Lipsett JA
Int J Radiat Oncol Biol Phys 1990 Jan;18(1):193-8. doi: 10.1016/0360-3016(90)90284-q. PMID: 2298622

Clinical prediction guides

Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis.
Tetikkurt C, Kubat B, Kulahci C, Tetikkurt S, Caliskaner Ozturk B
Monaldi Arch Chest Dis 2021 Apr 6;91(3) doi: 10.4081/monaldi.2021.1713. PMID: 33840184
Unilateral Upper Lung Field Pulmonary Fibrosis Radiologically Consistent with Pleuroparenchymal Fibroelastosis after Thoracotomy: A New Disease Entity Related to Thoracotomy.
Sekine A, Satoh H, Iwasawa T, Matsui K, Ikeya E, Ikeda S, Yamakawa H, Okuda R, Kitamura H, Shinohara T, Baba T, Komatsu S, Kato T, Hagiwara E, Ogura T
Respiration 2017;94(5):431-441. Epub 2017 Sep 1 doi: 10.1159/000479331. PMID: 28858871
Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease.
Enomoto Y, Nakamura Y, Colby TV, Johkoh T, Sumikawa H, Nishimoto K, Yoshimura K, Matsushima S, Oyama Y, Hozumi H, Kono M, Fujisawa T, Enomoto N, Inui N, Iwashita T, Suda T
PLoS One 2017;12(6):e0180283. Epub 2017 Jun 30 doi: 10.1371/journal.pone.0180283. PMID: 28666014Free PMC Article
Pleural and parenchymal fibrosis mainly affecting the upper lung lobes in persons exposed to asbestos.
Hillerdal G
Respir Med 1990 Mar;84(2):129-34. doi: 10.1016/s0954-6111(08)80015-7. PMID: 2371434
Spontaneous pneumothorax in patients irradiated for Hodgkin's disease and other malignant lymphomas.
Pezner RD, Horak DA, Sayegh HO, Lipsett JA
Int J Radiat Oncol Biol Phys 1990 Jan;18(1):193-8. doi: 10.1016/0360-3016(90)90284-q. PMID: 2298622

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